Independent of infection, in a model of misfolded mutant Superoxide dismutase (SOD1)-driven amyotrophic lateral sclerosis (ALS), damaged mitochondria-released mtDNA and mt(DNA/RNA) hybrids activated the cGAS-STING and DDX41-STING pathways, respectively (81). The gene discussed is STING1; the disease is amyotrophic lateral sclerosis.